Our examination of NR3 family member expression in a set of 22 DSRCT patients and three PDXs found high expression of glucocorticoid receptor (NR3C1) and an intriguing inverse relationship between AR expression and the expression of NR3C1. Future work examining the function of NR3C1 in DSRCT and its relationship with AR may provide greater insight into enzalutamide’s activity in DSRCT. Here, NR3C1 is linked to desmoplastic small round cell tumor.